Doctors at Delhi’s Sir Ganga Ram Hospital have removed a kidney weighing 7.4 kg from a 56-year-old man suffering from Autosomal Dominant Polycystic Kidney Disease (ADPKD). This is believed to be the “largest kidney” in India and the third-largest in the world. A human kidney on average weighs between 120-150 grams.
About:
What is it? Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the growth of numerous cysts in the kidneys. These cysts form inside the kidneys on the walls of hair-sized structures called nephrons, which help to filter out waste from the blood.
Symptoms: The most common symptoms include pain in the back and between the ribs and hips, headaches, blood in the urine, high blood pressure, and kidney insufficiency.
Cause:
ADPKD is caused by mutations in the PKD1 and PKD2 gene, that create proteins for the proper functioning of the kidneys and other parts of the body.
ADPKD is inherited as an autosomal dominant trait in families. This means that if one parent has the disease, there is a 50-percent chance that the disease will pass to a child of either gender.
Impact:
ADPKD is one of the most common causes of end-stage kidney disease (when kidneys can no longer function properly).
Though a kidney disease, ADPKD can affect other organ systems leading to a multisystem disorder. Organs that can be affected include the liver, pancreas, prostrate and glands of the male reproductive tract.
Treatment: Although there is no cure for ADPKD, treatment can ease symptoms and prolong life. Treatment includes dialysis and renal (kidney) transplant.
Vulnerable groups: ADPKD was earlier known as adult polycystic kidney disease, since it usually occurs in the fourth or fifth decade of life — but it has been reported in children and infants as well. Both men and women are equally likely to develop this disease.
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